Vaginal Agenesis Research Articles

Vaginal agenesis or distal vaginal atresia associated with anorectal malformations

BackgroundMayer-Rokitansky-Küster-Hauser (MRKH) syndrome is defined as Müllerian aplasia with vaginal agenesis and uterine remnants. It is commonly associated with renal and sometimes vertebral anomalies. The MRKH syndrome or distal vaginal atresia is sometimes associated with anorectal malformations. The purpose of this study was to describe 7 girls with vaginal agenesis or distal vaginal atresia and an anorectal malformation and review the literature. MethodsSeven patients with vaginal agenesis or distal vaginal atresia and anorectal malformation were operated on at 3 pediatric surgical centers in Madrid, Helsinki, and Stockholm. Case records were reviewed, and the previous literature was searched. ResultsSix patients had a reconstruction of the anorectum and vagina during the first year of life. In one case, the vagina was replaced at the time of a redo posterior sagittal anorectoplasty at the age of 11 years. The 4 patients with vaginal agenesis had a sigmoid colovaginoplasty. The 3 patients with a distal vaginal atresia had a vaginal pull-through. Four of the patients needed laxatives or enemas for mild constipation at last follow-up. Short-term gynecologic problems were minor in all patients. ConclusionVaginal reconstruction at the time of anorectoplasty results in good short-term outcome. For vaginal agenesis, a primary colovaginoplasty is suggested to be the preferred technique to replace the vagina.

MRI of Pregnancy-Related Issues: M??llerian Duct Anomalies

Müllerian duct anomalies can adversely affect pregnancy outcomes and can result in clinical symptoms. This article will review the appropriate management of patients with müllerian duct abnormalities. Whereas uterine and vaginal septa, vaginal agenesis, and unicornuate uterus can be managed surgically, other uterine anomalies tend to be managed clinically. Hence, appropriate management depends on a reliable assessment of pelvic anatomy. MRI can accurately display female pelvic anatomy and is, therefore, useful in guiding therapy.

Primary amenorrhoea in Kashmiri adolescent females – a hospital based study

Objectives: The basic aim of this study was to evaluate various causes of primary amenorrhea in Kashmiri adolescent females. Methods and material: the study was conducted in a tertiary care hospital on adolescent females with primary amenorrhoea in the age group of 13-19 years. A detailed history was taken and a thorough examination was done to note the secondary sexual characteristics. All cases were subjected to following investigations – haemoglobin, total leukocyte count, differential leukocyte count, erythrocyte sedimentation rate, coagulation profile and hormonal profile. Radiological investigations and karyotyping were done in selected cases. Statistical analysis: Done by descriptive analysis. Results: Eugonadism was the commonest type of primary amenorrhoea and was seen in 68 cases. Imperforate hymen was seen in 28 cases and complete vaginal atresia in 16. Among hypogonadotropic hypogonadism (23 cases) constitutional delay was seen in 15 cases, 8 cases diagnosed as hypogonadotropic hypogonadism was in fact having thyroid dysfunction. 3 cases with hypergonadotropic hypogonadism were seen and all were diagnosed as Turners syndrome. Conclusions: Though mullerian abnormalities are the most common cause of primary amenorrhoea, thyroid dysfunction should be taken as a possibility in evaluating primary amenorrhoea in an iodine deficient belt like Kashmir. Key words: adolescent; primary amenorrhoea; mullarian anomalies. DOI: http://dx.doi.org/10.4038/sljog.v32i3.3983 SLJOG 2010; 32(3): 53-56

Recurrent Vaginal Discharge in Children

BackgroundChildhood vaginal discharge remains a frequent reason for referral from primary to secondary care. The Pediatric and Adolescent Gynecology (PAG) service at Kettering General Hospital was established in 1993 and provides a specialized service that meets the needs of children with gynaecological conditions. AimTo investigate recurrent vaginal discharge noting symptomatology, defining pathogens, common and rarer causes, exploring management regimes, and any changes in practice over time. MethodRetrospective review spanning 15 years identifying prepubertal children attending the outpatient PAG clinic with recurrent vaginal discharge. We reviewed the medical notes individually. Results110 patients were identified; 85% were referred from primary care. The age distribution was bimodal at four and eight years. Thirty-five percent of our patients were discharged after the initial consultation. The commonest cause of discharge was vulvovaginitis (82%). Other important causes included suspected sexual abuse (5%), foreign body (3%), labial adhesions (3%), vaginal agenesis (2%). 35% of patients were admitted for vaginoscopy. ConclusionVaginal discharge is the most common gynecological symptom in prepubertal girls and can cause repeated clinical episodes. Vulvovaginitis is the most common cause and often responds to simple hygiene measures. Awareness of the less common causes of vaginal discharge is essential.

A Case of Vaginoplasty without Grafting

BackgroundMany techniques have been used to create a neovagina in patients with vaginal agenesis, and several surgical procedures involve the use of a graft. Grafting techniques are associated with intraoperative and postoperative morbidity and complications, at both the donor and the recipient site. CaseA 17-year-old patient with Mayer-Rokitansky-Kuster-Hauser syndrome complicated by unilateral pelvic kidney underwent successful vaginoplasty without the use of a graft, followed by insertion of a vaginal stent for 12 weeks. She had a functional, well-healed, and fully epithelialized neovagina by 6.5 months postoperatively. ConclusionPatients undergoing surgical vaginoplasty may benefit from this simple surgical technique, thereby avoiding the morbidity associated with the alternative grafting techniques commonly used in practice.

Management of the High Urogenital Sinus—Risk of Overexposure?

THE confluence of the female urethra with the vagina (urogenital [UG] sinus) into a single channel that opens in the vulva or perineum may result from the lack of reabsorption of the distal UG sinus (persistent UG sinus), from alterations in septation (persistent cloaca), or from the influence of endogenous or exogenous androgenic substances. 1 The distinction between the first 2 causes and the latter is crucial because it has a profound influence on the choice of surgical intervention needed to correct the anomaly. When the persistent UG sinus results from virilization, the distal part of the UG sinus persists, and the otherwise normal female urethra elongates and may indeed reach the glans clitoris. In these cases, representing the majority of girls with persistent UG sinus, simple caudal mobilization of the sinus and the vagina and urethra en bloc (total urogenital mobilization [TUM]) is effective, and does not affect urinary continence. 2‐4 In 1969 Hendren and Crawford advanced the concept that in some children with congenital adrenal hyperplasia (CAH) the confluence of the urethra and vagina can be located above what they called the external sphincter. 5 They suggested that performing a cut back of the sinus might result in incontinence, but this idea was based on endoscopic rather than anatomical observations. The anatomical validation for this concept was lacking. The observation that the vagina enters the verumontanum was incorrectly interpreted because in XX individuals there is never a verumontanum. What is often observed is a mound resembling the veru at the point of confluence. In practice we have never seen a patient with CAH become incontinent after TUM. The degree of virilization affects the length of the common channel distal to the confluence but not the length of the urethra proximal to it, which is always normal. 6 The advantages of TUM compared to techniques based on the separation of the urethra from the vagina are clear to anyone who has performed both procedures. It also makes anatomical sense since the urethral sphincter complex is omegashaped, incomplete posteriorly. 7 Separation of the urethra from the vagina, and not en bloc mobilization of the vagina and urethra, incurs the risk of damage to the continence mechanism. In our experience TUM can always be accomplished perineally with the patient in the dorsal lithotomy position. For the reasons mentioned we have completely abandoned techniques that separate the urethra from the vagina and have not observed any alterations in voiding or continence. 1,8 The situation is totally different in cases of UG sinus not associated with virilization. In these cases the urethra may be short and the surgical correction needs to be individualized. In addition, in these cases continence and voiding may be compromised by associated neurological abnormalities. 1 The anterior sagittal transrectal approach described by Salle et al in this issue of The Journal (page 1024) is based on the time-honored York-Mason operation for prostatorectal fistulas. 9 In children this approach can be useful in reoperations for membranous urethral strictures, high vaginal atresia when the uterus is present, posttraumatic situations and other challenging cases. 10 We recommend that pediatric urologists become familiar with this approach. Nevertheless, we consider it not only unnecessary but potentially harmful for the correction of UG sinus associated with virilization. In the report by Pippi Salle et al 1 girl with CAH needed a diverting colostomy because of a perineal infection. We applaud the technical expertise of the authors and are thankful to Dr. de Castro for having popularized this approach for pediatric urology. At the same time we urge caution, critical analysis of the published literature, and understanding of anatomy and function before embarking on new surgical adventures. Surgeons should tailor the magnitude of the operation to the needs of the patient and do no more than is needed to achieve the intended goal.

Pallister-Hall syndrome presenting as an intrauterine fetal demise at 39 weeks′ gestation

Pallister-Hall syndrome (PHS) is a pleiotropic autosomal-dominant malformation syndrome rarely presenting with genitourinary malformations. Literature has recorded 14 cases of PHS with genitourinary findings out of which only six have been females presenting with hydrometrocolpos and/or vaginal atresia. Fetal autopsy findings on a 39 weeks' gestation including demonstration of corticotroph deficiency in the pituitary, along with the review of literature is being presented here. None of the earlier literature pertaining to PHS with hydrometrocolpos and/or vaginal atresia describes an intrauterine fetal demise due to corticotroph deficiency.

Results of laparoscopic davydov technique in 47 patients with congenital absence of the vagina: laparoscopic peritoneal vaginoplasty

목적 본연구는 선천성 무질증 환자에서 복강경하에서 복막을 이용한 질재건수술 환자에서 수술 후 추적기간 동안 재건된 질의 해부학적 및 성기능을 연구한다. 연구방법 2005년과 2011년 사이에 연세의료원과 안양샘병원 산부인과에 내원한 47명의 무질증 환자(로키탄스키증후군)에서 복강경하에서 복막을 이용한 질재건술(laparoscopic Davydov technique)을 시행하였으며, 수술 후 3-12개월의 추적기간 동안 재건된 질의 길이, 넓이, 편평상피세포 전환과 세포성숙도를 알기위해 Schiller test을 시행하였다. 이 중 성교활동이 활발한 25명의 환자에서 성적만족도를 알기위해, 비슷한 년령의 일반대조군 기혼여성 15명과 성만족도(Female Sexual Function Index)를 비교조사하였다. 수술전 환자는 염색체검사, 호르몬검사, 및 MRI을 시행하였으며, 기타 비뇨기계 및 골격계기형유무를 알기위해 신우조영술 및 기타 방사선촬영을 시행하였다. 결과 평균수술 시간은 117 ± 35.3분이었으며, 수술 중 합병증으로서 2예에서 직장손상, 1예에서 방광손상이 있었으며, 그 이후 3-12개월 추적기간 동안, 경등도의 탈장 및 질육아조직미란이 각각 1예씩 관찰되었으며, 그 외 직장질루 또는 방광질루 등은 관찰되지 않았다. 추적환자의 평균 질길이는 8.3 ± 1.1 cm이었으며, 질의 넓이는 전환자에서 최소한 두 손가락(3 cm)이 통과되었으며, 성만족도는 일반 대조군과 차이가 없었다. 결론 복강경 Davydov 수술은 매우 안전하고, 합병증이 적으며, 해부학적 및 기능적으로도 우수한 결과를 얻었다. 이 수술법은 복강경하에서 최소침습시술을 함으로써 신체외부에도 보기 흉한 흉터를 남기지 않으며, 복막을 이용함으로써 자연적인 골반축을 유지할 수 있으며, 또한 자연질상피세포로의 전환이 용이하게 일어나 질건조나 위축이 적어 성적 만족도도 우수하였다. 또한 특별한 수술기구가 필요하지 않았으며, 부인과 의사가 단독으로 시행할 수 있는 좋은 수술법으로 생각된다.

Treatment of vaginal agenesis using a modified McIndoe technique: Long-term follow-up of 23 patients and a literature review

Treatment of vaginal agenesis using a modified McIndoe technique: Long-term follow-up of 23 patients and a literature review

Rectosigmoid Vaginoplasty: Clinical Experience and Outcomes in 86 Cases

There are several techniques for creation of a neovagina. However, rectosigmoid segment presents the most natural substitute for vaginal tissue. To evaluate the anatomical and functional results of sigmoid vaginoplasty and long-term sexual and psychological outcomes in 86 patients with vaginal absence. Between April 2000 and February 2009, 86 patients, aged 18 to 57 years (mean 22) underwent rectosigmoid vaginoplasty. Indications were vaginal agenesis (54), female transgenderism (27), and genital trauma (5). Rectosigmoid segments ranging from 8 cm to 11 cm were isolated, to avoid excessive mucus production. Preferably, it should be dissected distally first in order to check its mobility and determine the correct site for its proximal dissection. Stapling device was used for the colorectal anastomosis as the safest procedure. Creation of perineal cavity for vaginal replacement was performed using a simultaneous approach through the abdomen and perineum. Perineal skin flaps were designed for anastomosis with rectosigmoid vagina for the prevention of postoperative introital stenosis. Main Outcome Measures. Sexual and psychosocial outcomes assessment was based on the Female Sexual Function Index, Beck Depression Inventory, and standardized questionnaires. Follow-up ranged from 8 to 114 months (mean 47 months). Good aesthetic result was achieved in 77 cases. Neovaginal prolapse (7) and deformity of the introitus (9) were repaired by minor surgery. There was no excessive mucus production, vaginal pain, or diversion colitis. Satisfactory sexual and psychosocial outcome was achieved in 69 patients (80.23%). Rectosigmoid colon presents a good choice for vaginoplasty. According to our results, sexual function and psychosocial status of patients who underwent rectosigmoid vaginoplasty were not affected in general, and patients attained complete recovery.

Successful management of a thick transverse vaginal septum with a vesicovaginal fistula by vaginal expansion and surgery

We present a case of a thick transverse vaginal septum with a vesicovaginal fistula treated with vaginal expansion using dilators, followed by surgery. A 27-year-old woman was admitted to our hospital with vaginal atresia and cyclical hematuria. Urethroscopy and cystography showed a vesicovaginal fistula between the proximal vagina and bladder. The vaginal septum was 4 cm thick. Over a 6-month period, the distal vagina was expanded with the use of dilators and the septum was sufficiently thinned. Reconstruction using a transvaginal and transabdominal approach created a direct anastomosis between the proximal and distal vagina. No vaginal strictures or contractures in the anastomotic region have been observed during the 12 months following surgery. The combination of expansion and subsequent reconstructive surgery for the treatment of a thick transverse vaginal septum with a vesicovaginal fistula was less invasive than alternative surgical approaches and provided the desired outcome.

Vaginal Agenesis, the Hymen, and Associated Anomalies

Study Objectives Review anomalies in patients with vaginal agenesis. In particular, to clarify the impact of an absent hymen on the presence of other anomalies; on the success of creating a vagina with dilators; and on sexual function outcomes. Design Retrospective medical record review; questionnaire on sexual function. Setting Gynecology service at a children’s hospital and the practice of 1 gynecologist. Participants All patients with vaginal agenesis were identified from the databases, as well as the subgroup in which hymenal status was known. Outcome Measures Data regarding hymen, renal, skeletal, cardiac, and other anomalies; for women who had a neovagina, the technique used to create a functional vagina. Results Of 69 females (age range 2–70 years), renal tract anomalies (43.3%), vertebral anomalies (29%), cardiac anomalies (14.5%), and syndromes including Klippel-Feil (7%) and MURCS association (7%) were identified. Where hymenal status was known (n = 47), 31 were normal, and 16 had an absent hymen. Where the hymen was absent, renal agenesis was increased (odds ratio = 13.5, P < .001). There was no association between other anomalies and an absent hymen, or between the various anomalies. For women without a hymen, the likelihood of failing dilation therapy was increased (odds ratio = 21.7; P < .01]. Conclusion An absent hymen makes renal agenesis more likely and increases the likelihood that dilator techniques will fail. This condition appears to be associated with reports of long-term problems with poor lubrication that are potentially related to the absence of the peri-hymenal Bartholin’s glands.

Uterine Preservation and Vaginal Reconstruction in a Patient with Congenital Vaginal Agenesis Presenting with Cyclic Menouria

Study ObjectiveTo report a case with primary amenorrhea and cyclic menouria.DesignCase report.SettingTertiary academic care center.PatientsInterventionView Large Image Figure ViewerDownload Hi-res image Download (PPT)Measurements and Main ResultsThe fistula tract was completely resected. Vaginal reconstruction with sigmoid colon pedicled flap was performed. The proximal part of the neovagina was connected with the remnant cervix and a folley catheter was left in the uterine cavity for seven days in order to prevent obstruction. The patient has been menstruating regularly since the operation for the last eight months.ConclusionMenouria might be an early sign of congenital vesicouterine fistula. Uterine preservation might be considered in partial vaginal agenesis patients. Study ObjectiveTo report a case with primary amenorrhea and cyclic menouria. To report a case with primary amenorrhea and cyclic menouria. DesignCase report. Case report. SettingTertiary academic care center. Tertiary academic care center. Patients Intervention Measurements and Main ResultsThe fistula tract was completely resected. Vaginal reconstruction with sigmoid colon pedicled flap was performed. The proximal part of the neovagina was connected with the remnant cervix and a folley catheter was left in the uterine cavity for seven days in order to prevent obstruction. The patient has been menstruating regularly since the operation for the last eight months. The fistula tract was completely resected. Vaginal reconstruction with sigmoid colon pedicled flap was performed. The proximal part of the neovagina was connected with the remnant cervix and a folley catheter was left in the uterine cavity for seven days in order to prevent obstruction. The patient has been menstruating regularly since the operation for the last eight months. ConclusionMenouria might be an early sign of congenital vesicouterine fistula. Uterine preservation might be considered in partial vaginal agenesis patients. Menouria might be an early sign of congenital vesicouterine fistula. Uterine preservation might be considered in partial vaginal agenesis patients.

Pedicled thinned deep inferior epigastric artery perforator flap for perineal reconstruction: A preliminary report

Deep inferior epigastric artery perforator (DIEP) flap has proved to be an effective method in perineal reconstruction. However, a few literatures have reported thinned DIEP flap which yields a better functional and cosmetic result. There are also no clear guidelines on the degree to which a DIEP flap may be debulked of excess tissue before flap viability becomes compromised. In this preliminary report, a series of patients are presented whereby perineal reconstruction was achieved using the pedicled, thinned DIEP flap, based on debulking parameters from our clinical findings. Between September 2007 and August 2010, 12 pedicled, thinned DIEP island flaps for perineal reconstruction were performed on three patients with vulval or vaginal tumour, five patients with congenital vaginal agenesis and four patients with perineal Paget's disease. The flap was thinned in the plane inferior to the superficial inferior epigastric vein based on the subcutaneous vasculature of the abdominal wall, as depicted by preoperative computed tomography (CT) angiography (CTA) examination. Preoperative abdominal CTA can perfectly display the anatomy of DIEP flap. With a sensitivity of 100% and a specificity of 100% (Φ>0.5 mm), it helped in reducing the harvesting time for the flap and in guiding flap thinning. Partial necrosis of the distal flap occurred in a relatively large transverse flap measuring 24 cm×8.5 cm. One patient experienced dehiscence and a subsequent suture was successfully made. The other ten flaps were transplanted successfully without any complications. This series demonstrates that DIEP flap can be reliably debulked in the plane inferior to the superficial inferior epigastric vein with relatively no risk of necrosis and can be used safely in perineal reconstruction. The abdominal CTA can be employed as an assisting tool to plan the DIEP flap as well as guide flap thinning.

Management of Vaginal Agenesis

Rokitansky syndrome and complete androgen insensitivity syndrome are the most common causes of vaginal agenesis. Treatment should be deferred until adolescence to allow informed consent and compliance. The best treatment for vaginal agenesis remains controversial although vaginal dilation therapy is still widely considered the first line treatment because success rates are high and associated risks are low. A variety of surgical options are also available, each with enthusiastic proponents. Long-term outcome studies on most surgical techniques, however, are still lacking and until recently most studies have reported on success rate in terms of anatomical success only, without including sexual function. Moreover, the medical literature lacks prospective comparative outcome studies, meaning that current choice of surgical procedure relies greatly on the surgeon’s preference and experience.

Uterine Preservation and Vaginal Reconstruction in a Patient with Congenital Vaginal Agenesis Presenting With Cyclic Menouria

Herein we report the case of a patient with primary amenorrhea and cyclic menouria. The patient was a 20-year-old woman with primary amenorrhea and inability to achieve sexual intercourse. Clinical examination revealed normally developed labia majora and minora, clitoris, and external urethral orifice, but no vaginal opening. A mature female pubic hair pattern was present, and axillary hair development was normal. Breasts were normally developed. Abdominopelvic magnetic resonance imaging demonstrated a remnant upper vagina and unicornuate uterus filled with fluid, and left-sided renal agenesis. Intraoperatively, a congenital vesicouterine fistulous tract was observed. The fistulous tract was completely resected. Vaginal reconstruction using a sigmoid colon pedicled flap was performed. The proximal part of the neovagina was connected to the remnant cervix, and a Foley catheter was left in the uterine cavity for 7 days to prevent obstruction. The patient has been menstruating regularly since the operation. Menouria might be an early sign of congenital vesicouterine fistula. Resection of the fistulous tract with uterine preservation might be considered in patients with vaginal agenesis.

Laparoscope‐Assisted Creation of a Neovagina Using Pedicled Ileum Segment Transfer

To present our experience of vaginal reconstruction with the use of a pedicled ileum segment and laparoscope assistance, and to analyze its complications and long-term anatomic and functional results. The abdominal and perineal approaches were performed simultaneously with the patient in a special position. Under the guidance of laparoscopy, the target ileal segment was harvested and transposed down to the perineum through an artificial tunnel between the bladder and the rectum. A silicon vaginal tutor was introduced into the vaginal cavity and maintained all day long for 2-3 months. The complications and the anatomical and functional results were summarized and analyzed. From February 2002 to June 2010, 82 patients underwent laparoscope-assisted total vaginal reconstruction with a pedicled ileum segment at our department. Complications developed in 16 of 82 patients, including rectum and/or bladder injury during operation, acute renal failure, delayed healing of the ileocutaneous anastomosis, introital stenosis, and partial or complete intestinal obstruction. The abdominal cutaneous scar was acceptable after the surgery. The vulva was not altered, which was especially significant for patients with congenital vaginal atresia. The neovagina was patent, soft, moist, and flexible. The mean width and depth of the neovagina at the latest postoperative visit measured 3.2 and 15 cm, respectively. The favorable long-term anatomical and functional results demonstrate that our technique is ideal for patients with congenital vaginal atresia or patients who need secondary vaginal reconstruction. For the primary male-to-female transsexuals or hermaphrodites, it can be an alternative method for vaginal construction.

Uterus didelphys with unilateral vaginal atresia: multicystic dysplastic kidney is the precursor of “renal agenesis” and the key to early diagnosis of this genital anomaly

Uterus didelphys with obstructed hemivagina (UDWOH) and ipsilateral renal agenesis is a distinct but rare entity. To demonstrate the association between UDWOH and a multicystic dysplastic kidney (MCDK) in neonates. To demonstrate the usefulness of sonography with vaginal fluid instillation in the early and reliable diagnosis of this genital anomaly in girls with MCDK. Our patients were five female neonates with a MCDK, which had been detected in utero. We performed sonography of the internal genitalia in all patients before and after filling the vagina with saline. In all five girls, sonographic examination revealed uterus didelphys with obstruction of the vagina ipsilateral to the MCDK. In three girls, a dilated ureter originating from the MCDK and extending to the level of the vagina could be clearly demonstrated, with one of these including the ureteric insertion into the atretic vagina. MCDK is key to the early diagnosis of UDWOH and will facilitate the provision of appropriate treatment. The neonatal period provides a unique opportunity for detecting uterine anomalies by sonography. An MCDK usually involutes and eventually mimics renal agenesis later in life.

An observational study of women with müllerian agenesis and their need for vaginal dilator therapy

To assess vaginal development, sexual activity, and the efficacy of vaginal dilator therapy in women with vaginal agenesis (Mayer-Rokitansky-Kuster-Hauser syndrome). Retrospective case review. Tertiary referral university teaching hospital clinic for disorders of sexual development and differentiation (DSDD). Eighty cases of Rokitansky syndrome. None. Sexual activity and vaginal dimensions. The retrospective case review examined vaginal dimensions and sexual activity at presentation with further evaluation at completion of vaginal dilator therapy. Eleven of 80 patients had undergone surgery in the past; six of these 80 women had received dilator training elsewhere, and four were sexually active. Sixty-three of 80 patients had not undergone any previous treatment. Seventeen were having satisfactory sexual intercourse, 16 were having unsatisfactory sexual intercourse, and 26 had never been sexually active; for four women, no information had been recorded. A total of 32 patients underwent vaginal dilator treatment, and 25 completed the therapy. Their vaginal length increased from 3.2 cm (range: 0 to 7 cm) to 6.1 cm (range: 3 to 9 cm). Diagnosis and management of müllerian agenesis may be achieved without the need for surgery in the majority of cases. Dilator treatment for vaginal agenesis should be offered as first-line treatment, coordinated by a specialist nurse with input from a psychologist.

Satisfactory urethral coitus in a patient with vaginal stenosis: case report

Urethral coitus is a very rare sexual disorder seen in women either with intact or anomalous vagina such as vaginal agenesis or imperforate hymen. Only about 25 cases have been reported in the literature. We report a case of urethral coitus in a patient with surgically corrected ambiguous genitalia due to congenital adrenal hyperplasia. The patient had megalourethra and stenotic vaginal introitus. Megalourethra was corrected by plication technique. Correction of megalourethra with this technique has not been reported in literature up to now. To the best of our knowledge, the present case is the 26th of total cases with urethral coitus reported so far and the first case of urethral coitus associated with a stenotic vaginal introitus developing due to surgically corrected ambiguous genitalia.